User:Bzastrow/paroxysmal dyskinesia

This topic is being edited as an assignment in an undergraduate neurobiology course. The course is participating in the Wikipedia Education Program. The revised article will be posted by March 22, 2013. Please leave any comments on sources or information you would like to see on this topic.

General Outline

The plan for our page here is to outline the differences between the different types of paroxysmal dyskinesia. Since there is already a good amount of detail regarding the three individual types, we are going to focus on similarities and differences between them.

Clinical Signs and Symptoms

PKD

The symptoms for paroxysmal diskinesogenic choreoathetosis are varied from case to case, however, typically they consist of involuntary movements. Such contractile movements include dystonia, chorea, athetosis, and ballism. For example, “Her attacks were characterized as sudden unilateral stiffness of upper and lower limbs followed by an involuntary extrarotation of the arm and leg.” Another frequently occurring symptom is the presence of an aura before the attack. These sensations manifest in several forms, usually described as a tingling in the target limb.

A single limb is the most frequently attacked area; however it is possible for an attack to affect more than one limb. When more than one limb is affected, the two limbs are usually unilateral, even though cases of bilateral symptoms have also been observed. Another frequently affected area is the torso, with some patients of PKD twisting their body.

Attacks experienced by patients of PKD typically last less than a minute, however longer attack can occur. To further distinguish between PKD and epilepsy, patients typically retain consciousness during their attacks, and are able to recall the attacks even after they have ended. Despite retaining consciousness, patients are usually incapable of speech during the attack. The frequency of attacks vary greatly. Some patients have been noted as having 100s of attacks per day, while others go months without an attack.

PNKD

The attacks are comprised of dystonia, chorea, and athetosis just like PKD. As well these attacks are mostly of the limbs, and are usually unilateral or asymmetric. What sets PNKD apart from PKD is that the attacks can last anywhere from 4 minutes to 4 hours, but shorter and longer attacks have been reported as well.

Similar to PKD, attacks of PNKD are characterized by dystonia, chorea, and athetosis. The attacks also affect the limbs, usually unilaterally but bilateral symptoms have also been experienced. Patients of PNKD usually report the presence of an aura before an attack as well; however they are usually different from those of PKD patients. Once again the aura varies, but is typically felt in the target limb. Another frequently noted aura is dizziness

Patients of PNKD experience attacks that last much longer than those of PKD. These attacks vary in length and can last anywhere between 4 minutes and 4 hours. Similar to the difference between length of attacks, the intervals between attacks are much longer. The frequency of PNKD patients’ attacks last anywhere between 1 day to several months.

PED

PED attacks are characterized in multiple ways. One separating factor of them is that patients typically experience longer durations of dystonia during their attacks. The most frequent target of attacks are both legs bilaterally, rather than unilateral symptoms. The attacks have also been known to affect the upper half of the body as well. In some cases, patients have had attacks that affected the posturing of their neck and shoulder. Usually there is not an indicative aura symptom prior to a PED attack, which has to do with the nature of the onset of attacks.

The duration and frequency of PED attacks fall between PKD and PNKD. The attacks can be relieved with rest, typically taking about 10 minutes from cessation of the exercise. Attacks usually do not last longer than 30 minutes. Attacks usually occur somewhere between a day and a month, however, there is a great deal of variability here. This variability can be contributed to the nature of the onset of attacks.

Causes/Pathology

All PD associated subtypes have genetic contributions and are likely to run in a families genetic history due to dominant allele mutations. A dominant allele

Diagnosis

Diagnosis is similar, but slightly different for each type of PD. As well some types are more understood than others.

Paroxysmal Kinesigenic Dyskinesia (Paroxysmal Kinesigenic Choreoathethosis)

The guidelines for diagnosing Paroxysmal Kinesigenic Dyskinesia (also called Paroxysmal Kinesigenic Choreoathetosis) were reviewed and confirmed by Unterberger and Trinka. PKD consists of unexpected forms of involuntary movements of the body. These movements can include dystonia, chorea, and athetosis, which are all different sudden contractions of the muscles. These involuntary movements can be triggered by quick, sudden, but voluntary movements, such as standing up after sitting for awhile, running quickly, or being startled. Many of these PKD patients experience an aura-like sensation or a tingling in their limbs before any spasms occurs. The limbs, body, face, and neck can all be affected, however, the limbs are the most susceptible. As well the spasms usually only last about 1 minute or less, but longer attacks do occur. One of the most defining features about PD compared to epilepsy is that all the patients never lose consciousness during an attack. The patient is usually diagnosed sometime before their 20's. More likely diagnosed during childhood than early adulthood. Almost all PKD's are idiopathic, as well there is a familial history of autosomal dominant inheritance. Physical examination and brain imaging examinations show normal results, and an EEG shows no specific abnormality as well. However, the negative synchronous EEG results can be used to prove that PKD is not a sort of reflex epilepsy but a different disease.

Paroxysmal Non-kinesigenic Dyskinesia

Paroxysmal Non-kinesigenic Dyskinesia has a set guideline for diagnosis that is slightly different than PKD. PNKD usually occurs unexpectedly, and is not brought on by sudden movements or exercise. Instead the attacks are brought on by stresses such as emotional stress, fatigue, alcohol, or caffeine consumption. The attacks are comprised of dystonia, chorea, and athetosis just like PKD. As well these attacks are mostly of the limbs, and are usually unilateral or asymmetric. What sets PNKD apart from PKD is that the attacks can last anywhere from 4 minutes to 4 hours, but shorter and longer attacks have been reported as well. Just like PKD, PNKD also shows autosomal dominance in family history. Physical examination and brain imaging examinations show normal results, and EEG shows no specific abnormality as well.

Paroxysmal Exercise-induced Dyskinesia

Paroxysmal Exercise-induced Dyskinesia had a set guideline for diagnosis that is similar, but slightly different than both PKD and PNKD. PED attacks are comprised of dystonic and bilateral movements usually in the lower limbs of the body. These attacks are brought about only by exercise and physical exhaustion. PED patients do not feel an aura-like sensation before an attack occurs, unlike PKD and PNKD. As well, these attacks usually last from 5 to 30 minutes, and can occur once a day or once a month. Physical examination and brain imaging examinations show normal results, and EEG shows no specific abnormality as well.

References

1. Bhattacharyya, KB, S. Basu, AD Ray, and S. Bhattacharya. "Sporadic Paroxysmal Exercise Induced Dystonia: Report of a Case and Review of the Literature." PubMed. U.S. National Library of Medicine, n.d. Web. 27 Feb. 2013. <http://www.ncbi.nlm.nih.gov/pubmed/11146614>.
2. Crompton, Douglas E., and Samuel F. Berkovic. "The Borderland of Epilepsy: Clinical and Molecular Features of Phenomena That Mimic Epileptic Seizures." Science Direct. N.p., Apr. 2009. Web. 27 Feb. 2013. <http://www.sciencedirect.com/science/article/pii/S1474442209700596>.
3. Unterberger, Iris, and Eugen Trinka. "Diagnosis and Treatment of Paroxysmal Dyskinesias Revisited." PubMed. US National Library of Medicine National Institutes of Health, Sept. 2008. Web. 27 Feb. 2013. <http://www.ncbi.nlm.nih.gov/pmc/articles/pmc3002546/>.
4. Zhou, Jue-qian, Lie-min Zhou, Zi-yan Fang, Qian Wang, Zi-yi Chen, Li-bai Yang, Su-da Chen, and Xiao-dong Cai. "Analyzing Clinical and Electrophysiological Characteristics of Paroxysmal Dyskinesia." PubMed. US National Library of Medicine National Institutes of Health, 16 Jan. 2011. Web. 27 Feb. 2013. <http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3063430/>.